Abstract
Epithelioid angiosarcoma (EA) is an extremely rare subtype of angiosarcoma, which is characterized by large cells with an epithelioid morphology. EA typically arises in deep soft tissues, including the adrenal gland, skin and thyroid, however, EA rarely arises in the spine. The current study presents a case of osteolytic lesions involving the fourth lumbar (L4) level of the spine. Preoperatively, the patient was misdiagnosed with metastatic carcinoma, however, a radiological examination detected the presence of osteolytic or destructive lesions in the vertebrae, which extended into the pedicles. Histopathological and immunohistochemical evaluations were performed on the tumor tissue obtained from a decompression specimen of the L4 vertebra. A bone lesion composed of sheet-like malignant cells exhibiting atypical epithelioid morphology with vascular formation was observed. The presence of anastomosing vascular channels lined by epithelioid endothelial cells also indicated that focal endothelial differentiation had occurred. In addition, immunohistochemistry assays revealed that the lesion was positive for the endothelial cell markers, CD31, CD34 and vimentin. The tumor was treated with decompression of the L4 vertebra, followed by posterior stabilization. The patient subsequently refused chemotherapy and radiotherapy but completed six months of follow-up. At the time of writing, the tumor remains under control and the patient is asymptomatic. This case highlights the difficulty of diagnosing EA, which requires careful pathological examination and immunophenotype labeling. At present, CD31 is the most sensitive marker for detecting EA.
Highlights
Intraosseous epithelioid vascular tumors are generally classified as epithelioid hemangiomas (EHs), epithelioid hemangioendotheliomas (EHEs) or epithelioid angiosarcomas (EAs) [1,2,3,4]
The term EA refers to a variant of angiosarcoma which is characterized by tumor cells with an epithelioid morphology and these cells occasionally exhibit a pseudoglandular or alveolar arrangement [6,8]
EA has previously been recognized in extraosseous sites, only a few cases of this rare variant have been documented in the bone [5,10]
Summary
Intraosseous epithelioid vascular tumors are generally classified as epithelioid hemangiomas (EHs), epithelioid hemangioendotheliomas (EHEs) or epithelioid angiosarcomas (EAs) [1,2,3,4]. A knuckle deformity of the L4 vertebra was observed, as well as cystic lesions present on T1‐weighted images, which exhibited high intensities on T2‐weighted images (Fig. 1). Numerous brisk mitosis events exhibiting cellular pleomorphism were observed and in certain areas, irregularly dilated vascular formations adjacent to the solid tissue were detected. In the immunohistochemistry assays, tumor cells were positive for vimentin (Fig. 4), CD34 (Fig. 5) and CD31 (Fig. 6) expression and negative for the epithelial membrane antigen (EMA). These results supported an endothelioid‐related origin of the tumor
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